Harding ataxia

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Harding Ataxia

Harding Ataxia (pronounced: hahr-ding a-tak-see-uh) is a rare, inherited neurological disorder characterized by a slow, progressive degeneration of the cerebellum, the part of the brain responsible for coordinating movement.

Etymology

The term "Harding Ataxia" is named after the British neurologist, John Harding, who first described the condition in the late 20th century. The word "ataxia" comes from the Greek "a-", meaning "without", and "taxis", meaning "order". Thus, "ataxia" refers to the lack of order or coordination.

Symptoms

Harding Ataxia typically presents in early adulthood and progresses slowly over time. Symptoms may include:

  • Gait Ataxia: Difficulty with walking and balance
  • Dysarthria: Slurred or slow speech that can be difficult to understand
  • Nystagmus: Rapid, involuntary eye movement
  • Dysphagia: Difficulty swallowing

Causes

Harding Ataxia is caused by mutations in the ATXN1 gene. This gene provides instructions for making a protein that is involved in regulating other genes. Mutations in the ATXN1 gene disrupt the normal function of this protein, leading to the symptoms of Harding Ataxia.

Diagnosis

Diagnosis of Harding Ataxia is based on clinical examination, patient history, and genetic testing to identify mutations in the ATXN1 gene.

Treatment

There is currently no cure for Harding Ataxia. Treatment is symptomatic and supportive, and may include physical therapy, speech therapy, and medication to manage symptoms.

See Also

References

  • Harding, J. (1981). Classification of the hereditary ataxias and paraplegias. Lancet, 1(8224), 1151–1155.

External links

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